Department of Ophthalmology, University of Auckland, New Zealand.
Department of Ophthalmology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.
Clin Exp Ophthalmol. 2021 Mar;49(2):186-202. doi: 10.1111/ceo.13863. Epub 2021 Jan 10.
Neuromyelitis optica spectrum disorder (NMOSD) is an antibody-mediated inflammatory disease of the central nervous system that involves the optic nerves, spinal cord, and often other specific brain regions such as area postrema of the medulla. NMOSD was formerly classified as a variant of multiple sclerosis (MS), given the similar symptomatology and relapsing course but is now considered to have distinct clinical, paraclinical, immunological and prognostic features. The discovery of aquaporin 4 (AQP4) immunoglobulin G (IgG) has improved the ability to diagnose NMOSD. AQP4-IgG targets the astrocytic AQP4 water channel leading to complement activation and increased blood-brain barrier permeability. Accurate and early diagnosis is crucial as timely treatment may result in mitigation of long-term disability. Myelin oligodendrocyte glycoprotein (MOG)-IgG associated disorder (MOGAD) is a distinct nosologic entity, which has been more recently described. Its clinical spectrum partly overlaps that of seronegative NMOSD and MS. Although it is considered to have fewer relapses and better prognosis than NMOSD, the clinical course and outcome of MOGAD has not been fully characterized.
视神经脊髓炎谱系疾病（NMOSD）是一种累及视神经、脊髓且常累及延髓孤束核等特定脑区的中枢神经系统抗体介导的炎症性疾病。鉴于 NMOSD 与多发性硬化（MS）具有相似的临床症状和复发病程，NMOSD 曾被归为 MS 的一种变异型，但现在已被认为具有独特的临床、辅助检查、免疫和预后特征。水通道蛋白 4（AQP4）免疫球蛋白 G（IgG）的发现提高了 NMOSD 的诊断能力。AQP4-IgG 靶向星形胶质细胞 AQP4 水通道，导致补体激活和血脑屏障通透性增加。准确和早期诊断至关重要，因为及时治疗可能减轻长期残疾。髓鞘少突胶质细胞糖蛋白（MOG）-IgG 相关疾病（MOGAD）是一种新描述的明确疾病实体，其临床谱部分与抗 NMO 抗体阴性 NMOSD 和 MS 重叠。虽然 MOGAD 被认为比 NMOSD 复发少、预后好，但 MOGAD 的临床病程和结局尚未完全明确。
